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High serum concentrations of autoantibodies to HSP47 in nonspecific interstitial pneumonia compared with idiopathic pulmonary fibrosis

DOI: 10.1186/1471-2466-8-23

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Abstract:

We measured the serum levels of the autoantibodies to HSP47 in 38 patients with various forms of IIP [16 with idiopathic pulmonary fibrosis (IPF), 15 with idiopathic NSIP, 7 with cryptogenic organizing pneumonia (COP)] and 18 healthy volunteers.The serum levels of autoantibodies to HSP47 in patients with idiopathic NSIP were significantly higher than in patients with IPF (P < 0.01), COP (P < 0.05), and healthy volunteers (P < 0.05). In addition, those in fibrosing NSIP were significantly higher than those of cellular and fibrosing NSIP (p < 0.05).We found high levels of anti-HSP47 autoantibody titers in sera of patients with idiopathic fibrosing NSIP compared with other IIPs and healthy volunteers.The classification of idiopathic interstitial pneumonias (IIP) includes seven clinico-radiologic-pathological entities. Usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP) are the two largest subsets of IIP [1,2]. The distinction between NSIP and UIP is important for clinical decision-making because the prognosis is generally better and the response to corticosteroids and immunosuppressants is also better in patients with NSIP compared with UIP [3-7]. In addition, patients with cellular NSIP are reported to have excellent long-term prognosis, while the majority of patients with fibrotic NSIP die mostly within 5 to 10 years of diagnosis [6]. Because of these reasons, the distinction between cellular NSIP and fibrotic NSIP is also important.Clinicians often speculate on the presence of such pathological changes based on noninvasive imaging studies such as high-resolution computed tomography (HRCT) scans. However, the discrimination between NSIP and UIP cannot always be predicted accurately by HRCT. Although surgical (open or thoracoscopic) lung biopsy has been traditionally the ''gold standard'' for the diagnosis of interstitial lung diseases (ILD) and is clinically relevant for selection of appropriate therapy [8], it seems to be relatively inva

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